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Middle-aged women are significantly more frequently affected than men. The pathogenesis is currently not fully understood. Based on the presence of disease-specific autoantibodies, it is classified as an autoimmune liver disease, although a\u00a0combination of genetic predisposition and environmental factors contribute to disease development and progression.<\/jats:p>\n                  <jats:p>The diagnosis of PBC is based on a\u00a0cholestatic enzyme pattern together with the presence of anti-mitochondrial antibodies (AMA) or PBC-specific anti-nuclear antibodies (sp100, gp210). A\u00a0liver biopsy is rarely required to establish the diagnosis; exceptions are the suspicion of a\u00a0PBC autoimmune hepatitis (AIH) variant syndrome or the absence of the abovementioned antibodies.<\/jats:p>\n                  <jats:p>\n                    The therapeutic goal is to reduce cholestatic injury thereby preventing disease progression and to reduce symptoms. Approximately 60\u201370% of patients achieve clinical and biochemical remission with first-line treatment, i.e., ursodeoxycholic acid (UDCA). Recently, the therapeutic paradigm has shifted from achieving certain predefined response criteria to a\u00a0normalization of alkaline phosphatase (ALP) together with a\u00a0low-normal bilirubin level, as the latter was linked to improved outcomes in some subgroups. For patients who do not sufficiently respond to UDCA, the newly approved peroxisome proliferator-activated receptor (PPAR) agonists elafibranor and seladelpar, as well as bezafibrate (\n                    <jats:italic>off-label<\/jats:italic>\n                    use), should be used as a\u00a0combination treatment with UDCA. In patients with decompensated cirrhosis, liver transplantation has been associated with good long-term outcomes, albeit disease recurrence occurs in up to 50% by 15\u00a0years.\n                  <\/jats:p>","DOI":"10.1007\/s00508-026-02798-6","type":"journal-article","created":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T12:23:24Z","timestamp":1788956604000},"page":"831-858","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Austrian Society of Gastroenterology and Hepatology (\u00d6GGH) consensus on primary biliary cholangitis"],"prefix":"10.1007","volume":"138","author":[{"given":"Emina","family":"Halilbasic","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth","family":"Tatscher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elmar","family":"Aigner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lukas","family":"Burghart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivo","family":"Graziadei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Hametner-Schreil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedikt Silvester","family":"Hofer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harald","family":"Hofer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Maieron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattias","family":"Mandorfer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Peck-Radosavljevic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedikt","family":"Schaefer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Sieghart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Stradner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Traussnigg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Wagner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heinz","family":"Zoller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Fickert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Trauner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,9]]},"reference":[{"issue":"7650","key":"2798_CR1","doi-asserted-by":"publisher","first-page":"924","DOI":"10.1136\/bmj.39489.470347.AD","volume":"336","author":"GH Guyatt","year":"2008","unstructured":"Guyatt\u00a0GH, Oxman\u00a0AD, Vist\u00a0GE, Kunz\u00a0R, Falck-Ytter\u00a0Y, Alonso-Coello\u00a0P, et\u00a0al. 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E.\u00a0Tatscher served as a\u00a0speaker and\/or advisory board member for Gilead, Ipsen, Takeda, and Falk, and received travel support from Ipsen and Gilead. E.\u00a0Aigner served as speaker and\/or advisory board member for Sanofi-Aventis, Gilead, Eli Lilly, Boehringer, Bayer, Falk, Amicus, Takeda, and Roche, and received travel support from Sanofi-Aventis, Gilead, Eli Lilly, Boehringer, Bayer, Falk, Amicus, Takeda, and Roche. L.\u00a0Burghart received grant support from Gilead, served as a\u00a0speaker and\/or consultant and\/or advisory board member for Gilead, Ipsen and AbbVie, and received travel support from Eli Lilly, Gilead and Ipsen. I.\u00a0Graziadei served as a\u00a0speaker and\/or advisory board member for Gilead, Ipsen, AbbVie, Roche, Alfasigma, Intercept-Advanz; AstraZeneca and Falk, and received travel support from Gilead, Ipsen and Roche. S.\u00a0Hametner-Schreil served as speaker and\/or advisory board member for Falk, Ipsen, Gilead, Abbvie, Roche and received travel support from Roche, Abbvie and Gilead. B.S.\u00a0Hofer received travel support by Ipsen and Falk, speaking honoraria from Gilead and honoraria for educational materials from Ipsen. H.\u00a0Hofer served as a\u00a0speaker and\/or advisory board member for Abbvie, Falk, Gilead, Ipsen, Eli-Lilly, Boehringer-Ingelheim, and Madrigal, and received travel support from Abbvie, Falk, Gilead, Ipsen and Eli-Lilly. A.\u00a0Maieron served as a\u00a0speaker and\/or advisory board member for Gilead, Ipsen, Intercept; Astra Zeneca, Roche and Falk, and received travel support from Ipsen, Gilead and Roche. M.\u00a0Mandorfer received grant support from Echosens, served as a\u00a0speaker and\/or consultant and\/or advisory board member for AstraZeneca, Boehringer Ingelheim, Chiesi, Echosens, Eli Lilly, Ipsen, Madrigal, Takeda, and W.\u00a0L. Gore & Associates, and received travel support from Ipsen. M.\u00a0Peck-Radosavljevic served as speaker and\/or advisory board member for AstraZeneca, BMS, Boehringer-Ingelheim, Eli Lilly, Eisai, Falk, Gilead, Ipsen, Intercept-Advanz, Merz, MSD, Roche, Sanofi, Shionogi, and Sobi. B.\u00a0Schaefer received grant support from Astro Pharma and speaker fees from Pharmacosmos, Chiesi and Takeda, as well as travel support from Abbvie. M.\u00a0Stradner received grant support from Novartis, and Pharming and served as speaker and\/or advisory board member for AbbVie, Amgen, Astra Zeneca, BMS, Boehringer Ingelheim, CSL Behring, GSK, Janssen, Eli Lilly, GSK, Janssen, Eli Lilly, MSD, Novartis, Pfizer, Roche, Takeda, and UCB. He. S.\u00a0Traussnigg served as speaker and\/or advisory board member for Sanofi. M.\u00a0Wagner served as a\u00a0speaker and\/or advisory board member for Astra-Zeneca, Boehringer-Ingelheim, Ipsen, Madrigal, Roche, received grant support from Falk, and travel support from Falk, Gilead, Ipsen, Roche. H.\u00a0Zoller received grant support from Pharmacosmos, Novo Nordisk, served as speaker for Acino, Pierre Fabre, Falk, Menarini, Kedrion and as consultant for Novo Nordisk, Kedrion, Pharmacosmos, Pierre Fabre, Astra Zeneca, and received travels support from Pharmacosmos and Falk. P.\u00a0Fickert received grant support from Falk, lntercept, Janssen, Pliant, Gilead, and Ipsen, served as a\u00a0speaker and\/or consultant and\/or advisory board member for Sanofi-Aventis, Gilead, Merz, Takeda, Roche, Ipsen, and Falk. He is also co-inventor of patents on the medical use of 24-norursodeoxycholic acid (service inventions as employee) filed by the Medical University of Graz. M.\u00a0Trauner received grant support from Albireo, Alnylam, Cymabay, Falk, Genentech, Gilead, Intercept, MSD, Takeda and UltraGenyx, served as a\u00a0speaker and\/or consultant and\/or advisory board member for AbbVie, Albireo, Agomab, Alfasigma, Boehringer Ingelheim, BiomX, Chemomab, Dexoligo Therapeutics, Falk, Genfit, Gilead, GSK, Hightide, Intercept, Ipsen, Janssen, Madrigal, Mirum, MSD, Novartis, Phenex, Pliant, Rectify, Regulus, ProQR Therapeutics, Siemens and Shire; speaker fees from Albireo, Boehringer Ingelheim, Bristol-Myers Squibb, Falk, Gilead, Ipsen, Intercept, Madrigal, Mirum, and MSD, and received travel support from AbbVie, Falk, Gilead, Jannsen Intercept and Ipsen. He is also co-inventor of patents on the medical use of 24-norursodeoxycholic acid (service inventions as employee) filed by the Medical University of Graz. W.\u00a0Sieghart declares no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}}]}}